Interstitial lung disease
Combined pulmonary fibrosis and emphysema: effect of pulmonary rehabilitation in comparison with chronic obstructive pulmonary disease Hiromi Tomioka,1 Nobuaki Mamesaya,1 Shyuji Yamashita,1 Yoko Kida,1 Masahiro Kaneko,1 Hideki Sakai2
To cite: Tomioka H, Mamesaya N, Yamashita S, et al. Combined pulmonary fibrosis and emphysema: effect of pulmonary rehabilitation in comparison with chronic obstructive pulmonary disease. BMJ Open Resp Res 2016;3: e000099. doi:10.1136/ bmjresp-2015-000099
Received 29 June 2015 Revised 5 October 2015 Accepted 23 November 2015
1
Department of Respiratory Medicine, Kobe City Medical Center West Hospital, Kobe, Hyogo, Japan 2 Department of Rehabilitation, Kobe City Medical Center West Hospital, Kobe, Hyogo, Japan Correspondence to Dr Hiromi Tomioka; [email protected]
ABSTRACT Objective: To evaluate the effectiveness of short-term comprehensive inpatient pulmonary rehabilitation for patients with combined pulmonary fibrosis and emphysema (CPFE), and to compare responses with those of patients with chronic obstructive pulmonary disease (COPD) who underwent an identical programme. Design: Retrospective analysis of several outcome measures. Setting: Pulmonary ward at a 358-bed community teaching hospital. Methods: 3-week inpatient pulmonary rehabilitation programme assessed by pulmonary function tests, 6 min walk test and health-related quality of life (HRQL) using the Short Form-36 (SF-36). Results: 17 patients with CPFE and 49 patients with COPD were referred to and completed the programme between March 2007 and February 2015. Age, sex, smoking status, body mass index and the Medical Research Council dyspnoea grade were comparable between groups. In the CPFE group, improvement from the start of the programme to the programme end was observed in forced expiratory volume in 1 s (FEV1) (from 1.7±0.4 to 1.8±0.4, p=0.034); however, there was no significant improvement in the 6 min walk test (distance, SpO2 nadir and Borg scale on exercise). With regard to HRQL, improvement was observed in physical function ( p=0.015) whereas deterioration was observed in social functioning ( p=0.044). In the COPD group, significant improvement was observed after the programme in the FEV1, 6 min walk test and 4 of the 8 SF-36 subscales. There was a significant difference in changes in the 6 min walk distance: −16.6±58.4 in CPFE versus 30.2±55.6 in COPD ( p=0.009). In 2 domains, there was a significant difference in SF-36 scores between groups: Δvitality, −6.3±22.4 in CPFE versus 11.3±21.1 in COPD, p=0.009; and Δsocial functioning, −18.8±34.2 in CPFE versus 5.3±35.9 in COPD, p=0.027. Conclusion: Patients with COPD derived greater benefits than those with CPFE, from the relatively short periods of inpatient pulmonary rehabilitation.
KEY MESSAGES Patients with chronic obstructive pulmonary disease derived greater benefit from the inpatient pulmonary rehabilitation programme compared to patients with combined pulmonary fibrosis and emphysema (CPFE). Future research should focus on developing a pulmonary rehabilitation programme specifically tailored to the needs of patients with CPFE in an attempt to optimise the benefits of rehabilitation.
INTRODUCTION Pulmonary rehabilitation is a comprehensive intervention based on thorough patient assessment followed by patient-tailored therapies, which include but are not limited to exercise training, education and behavioural changes, and are designed to improve the physical and psychological condition of people with chronic respiratory disease, and to promote long-term adherence to health-enhancing behaviours.1 Pulmonary rehabilitation is an integral part of the clinical management of patients with chronic respiratory disease. Most of the evidence supporting the effectiveness of pulmonary rehabilitation and development of guidelines comes from studies of patients with chronic obstructive pulmonary disease (COPD) for which pulmonary rehabilitation has been found to reduce dyspnoea, increase exercise capacity and improve quality of life.2 Emphysema is the main pathological feature of COPD, which is characterised by the permanent abnormal enlargement of airspaces distal to the terminal bronchioles, accompanied by destruction of their walls. The characteristics of emphysema do not, by definition, include thickening of the alveolar septa and fibrosis. The occurrence of both emphysema and pulmonary fibrosis in the same patient has received increased
Tomioka H, Mamesaya N, Yamashita S, et al. BMJ Open Resp Res 2016;3:e000099. doi:10.1136/bmjresp-2015-000099
1
Open Access attention. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is a recently described entity associating upper-lobe emphysema with lower-lobe fibrosis.3 Given the evidence that pulmonary rehabilitation benefits a large population with COPD, it is a reasonable assumption that those with CPFE might benefit similarly. However, data on the effectiveness of pulmonary rehabilitation in patients with CPFE are scant. We therefore decided to elucidate the effectiveness of pulmonary rehabilitation in patients with CPFE in our cohort who participated in an inpatient pulmonary rehabilitation programme. The key question addressed in this study is whether pulmonary rehabilitation for patients with CPFE is as beneficial as it is for those with COPD.
MATERIALS AND METHODS Study participants Consecutive patients with CPFE and COPD without parenchymal lung disease with fibrosis who were referred to and completed the inpatient pulmonary rehabilitation programme at Kobe City Medical Center West Hospital, Kobe, Japan, between March 2007 and February 2015, were included in this study. Inclusion criteria were a stable clinical condition with no exacerbations in the preceding 2 weeks and written consent to participate in the programme. Patients with active cancer, unstable cardiac disease, or a history of neurological or orthopaedic disorders interfering with exercise, were excluded. CPFE was diagnosed when high-resolution CT (HRCT) chest scans showed emphysema occupying >25% of both upper lobes4 and idiopathic diffuse parenchymal disease with fibrosis of the lower lung zones.5 COPD was diagnosed and its severity assessed in accordance with the Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2014 guidelines.6 Patients with complicated parenchymal lung disease with fibrosis were excluded. Study design The study was a retrospective observational study of patients enrolled in our comprehensive pulmonary rehabilitation programme in a community hospital. Our institutional review board approved this retrospective study (reference number 15-001) and the requirement for informed consent was waived. Assessments Dyspnoea and functional status were evaluated using the Medical Research Council (MRC) dyspnoea grade (1– 5).7 Cumulative cigarette consumption expressed as the smoking index was the number of cigarettes consumed per day multiplied by years of smoking.8 Pulmonary function tests were assessed using a spirometer (Autospirometer System 7, Minato, Tokyo, Japan) according to the method described in the American Thoracic Society 1994 update.9 Predicted normal values 2
for the Japanese population were derived from reference values of the Japanese Respiratory Society.10 We reviewed HRCT scans of the participants. HRCT scanning was performed by helical CT scanners (Optima CT 660, GE Healthcare, Tokyo, Japan or SOMATOM Sensation 64, SIEMENS, Tokyo, Japan) with 1.0 mm-thick axial sections taken at 1 cm intervals throughout the entire thorax and were reconstructed using a high-spatial frequency algorithm. Emphysema was scored visually in the upper, middle and lower fields of both lungs according to the methods of Goddard et al.4 The score for each of the 6 lung fields was calculated according to the percentage of each lung field that was a lowattenuation area (%LAA): score 0 %LAA
Combined pulmonary fibrosis and emphysema: effect of pulmonary rehabilitation in comparison with chronic obstructive pulmonary disease Hiromi Tomioka,1 Nobuaki Mamesaya,1 Shyuji Yamashita,1 Yoko Kida,1 Masahiro Kaneko,1 Hideki Sakai2
To cite: Tomioka H, Mamesaya N, Yamashita S, et al. Combined pulmonary fibrosis and emphysema: effect of pulmonary rehabilitation in comparison with chronic obstructive pulmonary disease. BMJ Open Resp Res 2016;3: e000099. doi:10.1136/ bmjresp-2015-000099
Received 29 June 2015 Revised 5 October 2015 Accepted 23 November 2015
1
Department of Respiratory Medicine, Kobe City Medical Center West Hospital, Kobe, Hyogo, Japan 2 Department of Rehabilitation, Kobe City Medical Center West Hospital, Kobe, Hyogo, Japan Correspondence to Dr Hiromi Tomioka; [email protected]
ABSTRACT Objective: To evaluate the effectiveness of short-term comprehensive inpatient pulmonary rehabilitation for patients with combined pulmonary fibrosis and emphysema (CPFE), and to compare responses with those of patients with chronic obstructive pulmonary disease (COPD) who underwent an identical programme. Design: Retrospective analysis of several outcome measures. Setting: Pulmonary ward at a 358-bed community teaching hospital. Methods: 3-week inpatient pulmonary rehabilitation programme assessed by pulmonary function tests, 6 min walk test and health-related quality of life (HRQL) using the Short Form-36 (SF-36). Results: 17 patients with CPFE and 49 patients with COPD were referred to and completed the programme between March 2007 and February 2015. Age, sex, smoking status, body mass index and the Medical Research Council dyspnoea grade were comparable between groups. In the CPFE group, improvement from the start of the programme to the programme end was observed in forced expiratory volume in 1 s (FEV1) (from 1.7±0.4 to 1.8±0.4, p=0.034); however, there was no significant improvement in the 6 min walk test (distance, SpO2 nadir and Borg scale on exercise). With regard to HRQL, improvement was observed in physical function ( p=0.015) whereas deterioration was observed in social functioning ( p=0.044). In the COPD group, significant improvement was observed after the programme in the FEV1, 6 min walk test and 4 of the 8 SF-36 subscales. There was a significant difference in changes in the 6 min walk distance: −16.6±58.4 in CPFE versus 30.2±55.6 in COPD ( p=0.009). In 2 domains, there was a significant difference in SF-36 scores between groups: Δvitality, −6.3±22.4 in CPFE versus 11.3±21.1 in COPD, p=0.009; and Δsocial functioning, −18.8±34.2 in CPFE versus 5.3±35.9 in COPD, p=0.027. Conclusion: Patients with COPD derived greater benefits than those with CPFE, from the relatively short periods of inpatient pulmonary rehabilitation.
KEY MESSAGES Patients with chronic obstructive pulmonary disease derived greater benefit from the inpatient pulmonary rehabilitation programme compared to patients with combined pulmonary fibrosis and emphysema (CPFE). Future research should focus on developing a pulmonary rehabilitation programme specifically tailored to the needs of patients with CPFE in an attempt to optimise the benefits of rehabilitation.
INTRODUCTION Pulmonary rehabilitation is a comprehensive intervention based on thorough patient assessment followed by patient-tailored therapies, which include but are not limited to exercise training, education and behavioural changes, and are designed to improve the physical and psychological condition of people with chronic respiratory disease, and to promote long-term adherence to health-enhancing behaviours.1 Pulmonary rehabilitation is an integral part of the clinical management of patients with chronic respiratory disease. Most of the evidence supporting the effectiveness of pulmonary rehabilitation and development of guidelines comes from studies of patients with chronic obstructive pulmonary disease (COPD) for which pulmonary rehabilitation has been found to reduce dyspnoea, increase exercise capacity and improve quality of life.2 Emphysema is the main pathological feature of COPD, which is characterised by the permanent abnormal enlargement of airspaces distal to the terminal bronchioles, accompanied by destruction of their walls. The characteristics of emphysema do not, by definition, include thickening of the alveolar septa and fibrosis. The occurrence of both emphysema and pulmonary fibrosis in the same patient has received increased
Tomioka H, Mamesaya N, Yamashita S, et al. BMJ Open Resp Res 2016;3:e000099. doi:10.1136/bmjresp-2015-000099
1
Open Access attention. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is a recently described entity associating upper-lobe emphysema with lower-lobe fibrosis.3 Given the evidence that pulmonary rehabilitation benefits a large population with COPD, it is a reasonable assumption that those with CPFE might benefit similarly. However, data on the effectiveness of pulmonary rehabilitation in patients with CPFE are scant. We therefore decided to elucidate the effectiveness of pulmonary rehabilitation in patients with CPFE in our cohort who participated in an inpatient pulmonary rehabilitation programme. The key question addressed in this study is whether pulmonary rehabilitation for patients with CPFE is as beneficial as it is for those with COPD.
MATERIALS AND METHODS Study participants Consecutive patients with CPFE and COPD without parenchymal lung disease with fibrosis who were referred to and completed the inpatient pulmonary rehabilitation programme at Kobe City Medical Center West Hospital, Kobe, Japan, between March 2007 and February 2015, were included in this study. Inclusion criteria were a stable clinical condition with no exacerbations in the preceding 2 weeks and written consent to participate in the programme. Patients with active cancer, unstable cardiac disease, or a history of neurological or orthopaedic disorders interfering with exercise, were excluded. CPFE was diagnosed when high-resolution CT (HRCT) chest scans showed emphysema occupying >25% of both upper lobes4 and idiopathic diffuse parenchymal disease with fibrosis of the lower lung zones.5 COPD was diagnosed and its severity assessed in accordance with the Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2014 guidelines.6 Patients with complicated parenchymal lung disease with fibrosis were excluded. Study design The study was a retrospective observational study of patients enrolled in our comprehensive pulmonary rehabilitation programme in a community hospital. Our institutional review board approved this retrospective study (reference number 15-001) and the requirement for informed consent was waived. Assessments Dyspnoea and functional status were evaluated using the Medical Research Council (MRC) dyspnoea grade (1– 5).7 Cumulative cigarette consumption expressed as the smoking index was the number of cigarettes consumed per day multiplied by years of smoking.8 Pulmonary function tests were assessed using a spirometer (Autospirometer System 7, Minato, Tokyo, Japan) according to the method described in the American Thoracic Society 1994 update.9 Predicted normal values 2
for the Japanese population were derived from reference values of the Japanese Respiratory Society.10 We reviewed HRCT scans of the participants. HRCT scanning was performed by helical CT scanners (Optima CT 660, GE Healthcare, Tokyo, Japan or SOMATOM Sensation 64, SIEMENS, Tokyo, Japan) with 1.0 mm-thick axial sections taken at 1 cm intervals throughout the entire thorax and were reconstructed using a high-spatial frequency algorithm. Emphysema was scored visually in the upper, middle and lower fields of both lungs according to the methods of Goddard et al.4 The score for each of the 6 lung fields was calculated according to the percentage of each lung field that was a lowattenuation area (%LAA): score 0 %LAA
